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Mayer Rokitansky Kuster Hauser Syndrome (MRKH)

Mayer Rokitansky Kuster Hauser Syndrome (MRKH) is the complete failure in the development of the mullerian ducts, resulting in absence of the fallopian tubes, uterus, and most of vaginas (as 2/3rd of vagina is formed by Mullerian duct).


Karyotype = 46 XX


Phenotype = Female

Associated Abnormalities

  • Renal ectopy and agenesis (horse-shoe shaped kidney)
  • Skeletal abn abnormities (most common - scoliosis).
  • Cardiac anomalies.

Clinical Features

  • Patient present between 15-18 years of age with primary amenorrhea.
  • Secondary sexual characteristics are normal as ovaries are normal (because ovaries do not develop from mullerian duct but from genital ridge, so ovulation is also normal).
  • P/V = Vagina is felt like a blind pouch and uterus is absents,
  • Although in MRKH fallopian tube should be absent, typically a part of the distal tube is present."
  • Findings are confirmed by USG.


  • Repair of vaginal agenesis is done either by frank dilatation or vaginoplasty.
  • Vaginoplasty should only be performed when the girl is just married or about to be married.
  • Surgical management: Vaginoplasty either by McIndoe reed procedure or Williams vaginoplasty or amnion vaginoplasty.

Frank Dilatation


This non-surgical procedure consists of a woman applying gradual pressure with progressively increasing dilators over the mullerian pit for 15 minutes twice a day. An indentation is created by the end of 3 to 6 month. Some have satisfactory intercourse, but in many, vaginal size is inadequate and they need a surgical procedure eventually.

Differential Diagnosis

Testicular feminization syndrome.

Extra Edge:


Mullerian agenesis / MRKH syndrome (Genotype = 46XX)

Ovaries normal (As they develop from genital ridge)


Ovulation = Normal

Estrogen normal

Patient complains of 1O amenorrhea



Secondary sexual characteristic normal



Phenotype = normal female


MC Indoe vaginoplasty (Done just below or after marriage)

Mullerian dysgenesis


Resulting mullerian anomaly

Failure of fusion along whole length of Mullerian duct

Uterus didelphys (2 vagina, 2 cervix, 2 uterus)

Lower part of ducts fuse, leaving the cornua separate

Bicornuate uterus (always single vagina)


Uterus bicornis bicollis    Uterus bicornis unicollis

       2 cervix                    1 Cervix

       2 uterus                    2 Uterus

The ducts fuse along the entire length but resultant septum fails to dissolve

Septate uterus

The ducts fuse, septa disolves but the fundal bulge does not develop

Arcuate uterus

Both Mullerian ducts are absent

Mullerian agenesis

One Mullerian duct absent, only one present

Unicomuate uterus (Uterus, Cervix and Vagina though appear to be normal are only half of the fully developed organ)

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